Three investigators within the overall SCOR proposal require the use of cystic fibrosis mice for their individual components. Dr. Bedwell (Project 1) intends experiments with a G542X CF mouse, in order to test whether aminoglycoside antibiotics can activate read-through of this truncated CFTR in vivo. Dr. Benos (Project 3) intends to develop a transgenic mouse possessing the Ca2+ - activated C1- channel (CaCC) under the regulatory control of the fatty acid binding protein promoter in order to determine whether it is possible to rescue cystic fibrosis mice by activating an alternate C1- secretory pathway. Dr. Sorscher (Project 4) will use the CF Animal Core facility for studies of a delta F508 maturational agent (DMSO) in cystic fibrosis mice. The CF Animal Core is intended to expand and genotype colonies of cystic fibrosis mice and to assist SCOR investigators with development of these two new strains (G542X, CaCC) of CF mice. The Core will also assist with nasal potential difference (PD) measurements on CF and normal mice in order to test interventions designed to correct the CF defect. While the primary responsibility of the CF Animal Core will be to assist SCOR investigators, many other faculty on the UAB campus will benefit from the existence of a core facility designated to develop and breed CF mice.